essential steps

8/14/2019 Essential Steps

1/34

A Paediatric Sickle Cell ServicEssential Components

Dr Moira DickConsultant Community PaediatricianKings College HospitalLambeth PCT

1981-7 Belgrave Childrens Hospital/KCH1990- 2003 STH

1994- date KCH


2/34


3/34


4/34

Sickle cell diseasechronic condition

acute exacerbations

unpredictableseverity of pain can be unbearable

occasionally life threatening

extremely variablein UK many children of 1st generation

immigrant families


5/34

Essential steps main aim to ensure child grows into

self managing and coping adult


6/34

Essential stepsIdentify child

antenatal screening &counselling neonatal screening programme registration in paediatric clinic


7/34

Katys storyBorn 1992

3rd child2 elderbrothers

Screening buno antenatal

counselling


8/34

Aim of antenatal

screeningTo offer timely antenatal sickle cell and

thalassaemia screening to all women (and

couples) to facilitate informed decisionmaking

The offer includes the offer of, uptake of,reporting of prenatal diagnosis and any

subsequent action by the end of 12 weeksof pregnancy


9/34

South EastLondonSickle CellandThalassaemia Centre


10/34

Aim of neonatal screening

programmeTo achieve the lowest possible childhood

death rate and to minimise childhoodmorbidity from sickle cell disorders

Objectives:

Process tests in a timely manner

Identify and arrange timely follow up of

infants identified as needing furtherinvestigation

Offer treatment and start parentaleducation in a timely manner


11/34

Criteria for screening

programmesThe condition should be an important

health problem

The natural history of the condition shouldbe understood

There should be a simple, safe, preciseand validated screening test

There should be an effective treatmentand evidence of early treatment leading tobetter outcomes


12/34


3rd child2 elderbrothers

Screening butno antenatal

counselling

Neonatal

screening


13/34

Early treatment Penicillin prophylaxis

Conjugate pneumococcal immunisation

Education eg palpation of spleen,recognition of other signs

Offer of regular blood transfusions in thoseat risk of stroke ( transcranial dopplerscreening)


14/34

Pneumococcal

preventionGood evidence

Adherence

Practical considerations eg prescribing


15/34

Education & supportEvidence that added value in reducing

morbidity & mortality

Most evidence based on provision of carein clinic

setting


16/34

Blood transfusion for

children at risk of strokeGood evidence for transcranial doppler

scans in HbSS to identify those most at risk

Good evidence that blood transfusion canprevent the majority of strokes and canprevent recurrence


17/34

Newborn screening programmeWorking group

Executive summaryDetailed guidance

www.sickleandthal.org.uk
http://www.sickleandthal.org.uk/http://www.sickleandthal.org.uk/


18/34

Standards (1) Penicillin prophylaxis

ii) 90% of infants should have been

prescribed Penicillin V (or alternative) by3 months. 99% of infants should havebeen offered and prescribed Penicillin Vor alternative) by 6 months

iii) Any parental refusal should bedocumented


19/34

Standards (2) Pneumococcal immunisation

ii) 95% of infants should have completed

the primary prevenar ( conjugatedpneumococcal vaccine) course by 15months

iii) 95% should be given Pneumovax

(polysaccharide antigen) at 2 years ofage (24-27 months) and 5 yearlythereafter


20/34

Standards (3)Transcranial Doppler scanning (TCD)

90% of sickle cell centres should have

the capability of offering annual TCDs tochildren with SCD from the age of 3 yearsby 2008 and 99% should have thiscapability by 2010


21/34


3rd child2 elder

brothers

Screening butno antenatal

counselling

Neonatal

screening

ProphylaxisRegular follow

No TCD


22/34

Katyaged 5 (1997) had TIA & then acute strokeright hemiplegiaMRI cerebral infarct left hemisphere

TCD showed raised velocities left MCAOn transfusion programmemild learning difficulties

Adams RJ, McKie VC, Hsu L, et al. Prevention of a

first stroke by transfusions in children with sickle cell

anemia and abnormal results on transcranial Doppler

ultrasonography. N Engl J Med. 1998;339:5-11.


23/34


24/34

Aspects of outpatient

management Primary and secondary prevention

Recognition of acute/chronic problems

Screening for complications Specific treatments eg Hydroxyurea, BMT


25/34

Other concernsimmigration

housing

schooling

future pregnancies

admissions to hospital/time off work

travel

+ all other complications


26/34

Essential stepsFollow up

minimum standards for out patientseducation & support

guidelines for acute complications

comprehensive but flexible

meets individual and familys needs

procedure for those lost to follow up

transition to adult service


27/34


28/34

Psychological support*painful, chronic and life threatening

condition

nocturnal enuresis commongood evidence for cognitive behavioural

therapy

necessary for assessing children who areat risk or who have had stroke

*very few units have clinical or health

psychology


29/34

Acute managementmajority of care is ambulatoryif get this right, inpatient admissions will be

very few

need to have local protocolsdetailed guidance is available

pathways for transfer eg to PICU

care plans for frequent attenders + thoseapproaching transition

www.sickleandthal.org.uk
http://www.sickleandthal.org.uk/http://www.sickleandthal.org.uk/


30/34

Standards (4)Failsafe arrangements

ii)the sickle cell centres in conjunction with

local paediatric units should havecontinuing responsibility for children withSCD identified on the newborn screeningprogramme and should maintain a list

iii)by 2008 95% ( 99% by 2010) should haverobust FU arrangements and the capabilityto track children


31/34

Organisation of servicePrimary careCommunity clinicsHospital clinics

Paediatric A/EPaediatric wards /day units for transfusionSickle cell centres inreach/outreachFollow up for DNA

Good liaison between primary/community/acute general & specialistCare pathwaysMultidisciplinary team


32/34


33/34

Role of local unit Role of sickle

centreNamed paediatrician Named paediatrician orhaematologist

Routine OP Designated clinic

Annual review/TCDHydroxyurea/BMT

Acute management of

painful episode/anaemia

Management of

ACS/strokeProvision HDU/PICU

Follow up Register/audit/research/

training


34/34

Final thoughts the sort of paediatric service to be set up will

depend on numbers of children , expertise ofstaff and available resource and geography

paediatricians (acute & community) are keyto its success and training needs for juniorstaff recognised

specialist nurses and psychologists areessential

sickle cell centres still need to be developed

availability of TCD not yet universal

essential steps

Documents